Ectodermal dysplasia 165

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tion commonly occurs during unfavorable conditions, such as poverty, war, or captivity.

Cause

The infection is caused by group A streptococci or Staphylococcus aureus.

Symptoms

The condition begins with one lesion, which enlarges and encrusts; beneath this crust is a pus-filled "punched ulcer." Children are more commonly affected with ecthyma, which is usually associated with poor hygiene and malnutrition and minor skin injuries from trauma, insect bites, or scabies.

Treatment

Erythromycin or dicloxacillin is administered. The lesions should also be soaked and crusts removed.

ectodermal dysplasia (ED) A group of genetic disorders that are identified by the absence or deficient function of at least two of these: teeth, hair, nails, or glands. At least 150 different EDs have been identified. Charles Darwin identified the first EDs in the 1860s, and today the number is believed to affect as many as seven out of every 10,000 births.

There is usually no reason to expect anything but normal intelligence with ectodermal dysplasia, though some of the extremely rare forms have been associated with mental retardation. As with the general population, some individuals affected by ED may be very bright, some may be average, and others may find challenges in learning.

Symptoms

Individuals affected by EDs have abnormalities of sweat glands, tooth buds, hair follicles, and nail development. Some types of EDs are mild while others are devastating. Many individuals affected by EDs cannot perspire. Air conditioning in the home, school, and workplace is a necessity. Other symptoms may include deficient tears and saliva, frequent respiratory infections, poor hearing or vision, missing fingers or toes, cleft lip and/or palate, poor immune system, sensitivity to light, and lack of breast development.

Life span can be affected in some rare types of EDs, but there are very few documented examples of a death because of an inability to perspire.

There are four different types of ectodermal dys-plasia: anhidrotic, anhydrotic, hypohidrotic, and hypohydrotic.

• anhidrotic means "no sweating" and is derived from the Greek words an ("none") and hidros ("sweat"). A person who does not sweat at all could be said to be anhidrotic.

• anhydrotic means "no water" from the Greek an ("none") and hydro ("water"). Those who are totally devoid of water could be said to be anhydrotic.

• hypohidrotic means "deficient sweating" from the Greek hypo ("under/deficient") and hidros ("sweat"). Someone whose sweat function is diminished (sweats little in response to heat or in response to stress only) could be said to be hypohidrotic.

• hypohydrotic means "deficient water." Those who are partially or totally devoid of water could be said to be hypohydrotic.

Cause

As a baby is developing, three layers of tissue can be identified: an inner layer (the endoderm), a middle layer (the mesoderm), and an outer layer (the ectoderm). Defects in formation of the outer layer lead to ED. The reason that so many parts of the body are affected is because the ectoderm of the surface of the developing baby forms the skin, nails, hair, sweat glands, parts of the teeth, the lens of the eye, and the parts of the inner ear. Another portion of ectoderm forms the brain, spinal cord, nerves, the retina of the eye, and the pigment cells of the body.

Treatment

At this time, there is no cure. Because most people with EDs have missing or malformed teeth, dental treatment is necessary beginning with dentures as early as age two, multiple replacements as the child grows, and perhaps dental implants thereafter. Orthodontic treatment may also be necessary.

Precautions must be taken to limit upper respiratory infections, and care must be provided for the skin to prevent cracking, bleeding, and infection.

Professional care may minimize the effects of vision or hearing deficits, and surgical or cosmetic procedures may improve other deformities.

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